Fibrodysplasia Ossificans Progressiva: treatment and oral considerations

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare disease with a worldwide prevalence of one in two million individuals. Heterotopic ossification is the main characteristic of this disease, where non-osseous tissues are able to form bone spontaneously or caused (blows, infections, intramuscular punctures). Consequently, affected patients progressively lose mobility and see themselves "trapped" in a second skeleton. The average life of these patients is about 40 years and, to avoid the rapid progression of the disease, several measures have to be taken in daily life. The objective of this work is to review the literature in search of information about FOP, its treatment and, mainly, oral considerations in patients with this disease. Some considerations are drawn up in different approaches in dentistry: craniomandibular alterations, medication, anesthesia, conservative dentistry, surgery and orthodontics. The information obtained is mainly based on isolated clinical cases or series of clinical cases. In spite of the limited information available, the conclusion is that treatment and dental management of these patients have to be approached by a multidisciplinary and trained team. The main oral complication in these patients is ankylosis of the temporomandibular joint (TMJ) that leads to nutrition problems and difficult access to the oral cavity. The function of the dentist is to improve the life quality of these patients by always avoiding actions that worsen their situation and cause an episode of ossification. The technique of truncal anesthesia and procedures that require excessive stretching of the TMJ are totally contraindicated. However, there is not enough scientific evidence, and more studies of dental management should be performed on these patients.